Medical Care

See the list below:

If a child with Hirschsprung disease has symptoms and signs of a high-grade intestinal obstruction, initial therapy should include intravenous hydration, withholding of enteral intake, and intestinal and gastric decompression.

Decompression can be accomplished through placement of a nasogastric tube and either digital rectal examination or normal saline rectal irrigations 3-4 times daily.

While awaiting surgical intervention in the event of a planned single-stage pull-through procedure, the baby should receive scheduled vaccinations.

Next:

Surgical Care

The surgical options vary according to the patient's age, mental status, ability to perform activities of daily living, length of the aganglionic segment, degree of colonic dilation, and presence of enterocolitis.

Surgical options include leveling colostomy, which is a colostomy at the level of normal bowel; a staged procedure with placement of a leveled colostomy followed by a pull-through procedure; or a single-stage pull-through procedure. The single-stage pull-through procedure may be performed with laparoscopic, open, or transanal techniques. This procedure can be performed at the time of diagnosis or after the newborn has had rectal irrigations at home and has passed the physiologic nadir. Colostomy followed by pull-through procedure is generally reserved for those patients who present with sepsis due to enterocolitis, massive distention of ganglionic bowel prohibiting pull-through procedure, or are otherwise not medically suitable for the pull-through procedure.

The ability to perform a single-stage pull-through procedure largely depends on the availability, experience, and capabilities of the staff pathologist because aganglionic intestine must not be in the pull-through segment.

Disclosure: Received honoraria from Prometheus Laboratories for speaking and teaching; Received honoraria from Abbott Nutritionals for speaking and teaching. for: Abbott Nutritional, Abbvie, speakers' bureau.